Cochleovestibular Nerve Research Articles

The "Near"-Narrowed Internal Auditory Canal Syndrome in Adults: Clinical Aspects, Audio-Vestibular Findings, and Radiological Criteria for Diagnosis.

Vestibular Paroxysmia (VP) refers to short attacks of vertigo, spontaneous or triggered by head movements, and implies the presence of a compressive vascular loop in contact with the cochleovestibular nerve (CVN). Classically, a narrowed internal auditory canal (IAC) corresponds to a diameter of less than 2 mm on CT, usually associated with a hypoplastic CVN on MRI. The aim of this study was to discuss a distinct clinical entity mimicking VP in relation to a "near"-narrowed IAC (NNIAC) and to propose radiological criteria for its diagnosis. Radiological measurements of the IAC were compared between three groups: the study group (SG, subjects with a clinical presentation suggestive of VP, but whose MRI of the inner ear and pontocerebellar angle excluded a compressive vascular loop) and two control groups (adult and children) with normal vestibular evaluations and no history of vertigo. 59 subjects (18 M and 41 F) were included in the SG. The main symptoms of NNIAC were positional vertigo, exercise- or rapid head movements-induced vertigo, and dizziness. The statistical analysis in the study group showed that the threshold values for diagnosis were 3.3 mm (in tomodensitometry) and 2.9 mm (in MRI) in coronal sections of IAC. Although a significantly lower mean value for axial IAC diameter was found in SG compared with controls, the statistics did not reveal a threshold due to the large inter-individual variations in IAC measurements in normal subjects. There was no significant difference in IAC diameter between the adult and pediatric controls. In the present study, we report a new anatomopathological condition that appears to be responsible for a clinical picture very similar-but not identical-to VP in association with the presence of an NNIAC. The diagnosis requires a careful analysis of the IAC's shape and diameters in both axial and coronal planes.

Typewriter Tinnitus: Value of ABR as a Diagnostic and Prognostic Indicator.

Typewriter tinnitus refers to a special kind of staccato tinnitus, which is mostly described by patients as Morse code, popcorn, or machine-gun. It has been accepted that the mechanism of typewriter tinnitus is caused by the neurovascular compression of the cochleovestibular nerve. Patients who suffered from typewriter tinnitus have exhibited a good response to carbamazepine or oxcarbazepine, but there is a risk of recurrence after treatment cessation. The present study aims to determine the value of auditory brainstem response (ABR) in diagnosing typewriter tinnitus and predicting relapse after drug withdrawal. Patients who presented with typewriter tinnitus from March 2019 to March 2022 were included for the present retrospective study. The auditory and vestibular test results and drug treatment effects were collected and analyzed. Patients with idiopathic unilateral subjective tinnitus, who were matched by age to patients with typewriter tinnitus at a ratio of 2:1, were consecutively recruited for the control group. Eighteen patients with typewriter tinnitus and 38 controls were included. Ears with typewriter tinnitus had longer interpeak latency (IPL) I-III, and wave III and V latencies, and a higher ratio of IPL I-III ≥2.3 ms based on ABR, when compared to the unaffected side and controls ( p <0.05). Seventeen patients with typewriter tinnitus responded positively to medication. Among these patients, seven patients had a relapse after drug cessation, while 10 patients did not have a relapse. The relapse group had significantly longer IPL I-III and wave V latency, older age, and poorer hearing, when compared to the nonrelapse group ( p < 0.05). Furthermore, IPL I-III had the largest area under the receiver operating characteristic curve, and the optimal cutoff was 2.4 ms (sensitivity, 100.0%; specificity, 66.7%). There were no significant differences in other demography or other clinical test results between the relapse and nonrelapse groups ( p > 0.05). Ramsay Hunt syndrome and neuromyelitis optica spectrum disorders were identified in two cases. Prolonged IPL I-III based on ABR can help in the diagnosis of typewriter tinnitus and its prognosis after treatment cessation. Patients with IPL I-III greater than 2.4 ms, older age and poorer hearing are more likely to relapse. In addition to the neurovascular conflict of the cochleovestibular nerve, the etiologies of neuroinflammation and demyelinating diseases are also possible for typewriter tinnitus.

Vestibulo-Cochlear Symptoms Revealing Vertebro-Basilar Dolicho-Ectasia

Introduction: Vertebrobasilar dolichoectasia (VBD) are uncommon arteriopathies defined by increased length and caliber of one or more intracranial arteries. The clinical picture is polymorphic. They are most often associated with ischemic or hemorrhagic stroke, but this pathology can also cause symptoms of cranial nerve compression. Objective: We report the case of a vertebro-basilar dolichoectasia collected in the radiology department of Ibn Tofail Hospital in Marrakech, revealed by vestibulo-cochlear symptoms and diagnosed by brain MRI. In the light of this observation, we review this uncommon pathology and the contribution of magnetic resonance imaging in its diagnosis. Case report: The patient was 68 years old and had a history of severe sleep apnea syndrome. He presented with rotatory vertigo, tinnitus and hypoacusis on the left side of the head for 3 months. The neurological examination was normal and the examination of the ENT sphere revealed bilateral sensorineural hearing loss and left hyporeflexia. A cerebral MRI was performed as part of the etiological workup, which revealed elongation and dilation of the vertebral arteries measuring 3.9 mm on the left and 5.2 mm on the right, with loops of which the left crossed the homolateral cochleovestibular nerve 7 mm from its emergence, responsible for a deviation of its path, and the glossopharyngeal nerve 4 mm from its emergence. It also showed an elongation and dilatation of the basilar trunk measuring 6.8 mm, presenting a loop lateralized to the left at the level of its origin (Smoker's Grade 2) with a bifurcation opposite the floor of the V3 (Smoker's Grade 2).Thus, the diagnosis of a DEVB responsible for a vascular-nervous conflict was retained. The indication for surgical management was given but was refused by the patient due to the deep location of the arteriopathy. Discussion and conclusion: DEVB is a rare and progressive condition with cranial nerve compression as a possible complication. Brain MRI is...

FEATURES OF NEUROLOGICAL DISORDERS IN CHILDREN WITH CONGENITAL AND ACQUIRED SENSORINEURAL HEARING LOSS

Sensorineural hearing loss is currently the most important medical and social problem and is an urgent problem of neurology. According to various researchers, from 6% to 36% of the worlds population suffers from ear pathology, of which 60-80% have sensorineural hearing impairment (damage to the cochleo-vestibular nerve receptor - the cochlea of the inner ear).Deafness is the most common defect in sensory systems in children and occurs in 1: 750 cases, and in adults 4-36% depending on age, which reflects the interaction of environmental factors and individual genetic predisposition (Tranebjaerg L., 2000).About 400 genetic syndromes that include hearing impairment have been described (Friedman T.V., Griffith A J., 2003). KEY WORDS: sensorineural hearing loss, neurological disorders. Purpose: to study clinical and neurological features in patients with sensorineural hearing loss.

Labyrinthitis With Negative MRI As a Precursor to Rapidly Developing Primary CNS Lymphoma of the Cerebellopontine Angle.

Few case reports have described primary central nervous system lymphoma (PCNSL) presenting as a cerebellopontine angle (CPA) lesion in HIV-positive patients. We describe a rare presentation of rapidly progressing PCNSL of the CPA/internal auditory canal (IAC) as labyrinthitis with initial negative MRI in an HIV-positive patient. A 58-year-old male with well-controlled HIV presented with sudden left sensorineural hearing loss, tinnitus, and imbalance. Vestibular testing suggested an uncompensated left peripheral vestibular weakness. MRI demonstrated facial and cochleovestibular nerve enhancement within the CPA and IAC. The presumptive diagnosis of labyrinthitis was made. Two months later, he presented to his infectious disease provider with left facial weakness and disequilibrium and was treated for presumed Bell's palsy. One month later, he presented with left corneal reflex dysfunction, decreased visual acuity, diplopia, and worsening ataxia. Repeat MRI demonstrated a new 3.6 cm lesion of the left CPA/IAC with vasogenic edema. Despite location, the mass lacked the brainstem compression characteristic of other extra-axial CPA masses such as vestibular schwannoma. Flow cytometry and cytology from cerebrospinal fluid was consistent with primary central nervous system large B-cell lymphoma. We present a unique case of rapidly progressing PCNSL of the CPA/IAC in an HIV-positive patient, presenting initially as labyrinthitis with negative MRI followed by development of multiple cranial neuropathies and 3-month repeat MRI demonstrating a large CPA mass. In HIV-positive patients with a similar initial presentation, PCNSL should considered early in the diagnostic evaluation with close clinical monitoring and a low threshold for repeat imaging.

Vestibular Neuritis after COVID-19 Vaccination

Coronavirus disease 2019 (COVID-19) spread rapidly and was declared a pandemic. In this situation, vaccination for COVID-19 is important and has been prompted in many countries. However, a number of adverse events have been reported for the Pfizer, Moderna, and AstraZeneca vaccines. Otolaryngologic adverse events after COVID-19 vaccination were reported, including several cases of sudden sensorineural hearing loss. Vestibular neuritis (VN) is an acute vestibular syndrome that causes acute and spontaneous vertigo due to unilateral vestibular deafferentiation, leading to nausea or vomiting and unsteadiness that can last from days to weeks. Neurotropism for the cochleovestibular nerve, immunoglobulin G mediated immune off-target reaction, and reactivation of latent viruses are hypothesized to be etiologies. Herein, the case of a 49-year-old female who developed VN after COVID-19 vaccination is presented. Although the association between VN and COVID-19 vaccination remains unclear, clinicians should be aware that VN may occur as an adverse event of COVID-19 vaccination.

Effect of AR42 in Primary Vestibular Schwannoma Cells and a Xenograft Model of Vestibular Schwannoma.

AR42, a histone deacetylase (HDAC) inhibitor, reduces viability of primary vestibular schwannoma (VS) cells and delays tumor progression and hearing loss (HL) in a xenograft model of VS. The impact of HDAC expression on AR42 response in primary VS cells is unknown, as well as the effects of AR42 on VS-associated HL and imbalance. Primary human VS cells (n = 7) were treated with AR42 (0-3.0 μM), and viability assays were conducted. Immunohistochemistry and western blotting for phosphorylated-HDAC2 (pHDAC2) were performed on tumor chunks. Pharmacokinetic studies were conducted in Fischer rats using mass spectrometry. Merlin-deficient Schwann cells were grafted onto cochleovestibular nerves of immunodeficient rats and treated with vehicle (n=7) or AR42 (25 mg/kg/day for 4weeks; n=12). Tumor bioluminescence imaging, auditory brainstem response (ABR), and rotarod tests were conducted to 6weeks. Final tumor weight and toxicities were measured. AR42 caused dose-dependent reductions in viability of VS cells. Tumors with higher pHDAC2:HDAC2 ratios had greater reductions in viability with AR42. On pharmacokinetic studies, AR42 reached peak levels in nerve ~24 hours after oral administration. Although AR42-treated rats demonstrated mean ABR threshold shifts ~10 to 20 dB lower than controls, this did not persist nor reach significance. When compared to controls, AR42 did not affect tumor bioluminescence, tumor weight, and rotarod measurements. Response of primary VS cells to AR42 may be influenced by pHDAC2 expression in tumor. Although AR42 may delay HL in our xenograft model, it did not halt tumor growth or vestibular dysfunction. Further investigations are warranted to evaluate the AR42 effectiveness in NF2-associated VS.

Table of contents

The LaryngoscopeVolume 132, Issue S8 p. v-v Issue InformationFree Access Table of contents First published: 14 May 2022 https://doi.org/10.1002/lary.30146AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article. Volume132, IssueS8Supplement: Can Imaging Predict Hearing Outcomes in Children with Cochleovestibular Nerve Abnormalities?June 2022Pages v-v RelatedInformation

X-linked deafness/incomplete partition type 3: Radiological evaluation of temporal bone and intracranial findings.

X-linked deafness (XLD) is a rare disease, characterized by typical cochlear incomplete partition type 3 anomaly (IP-III). Accompanying hypothalamic anomalies were also recently described. The purpose of this study was to document the temporal bone and intracranial imaging findings in a series of patients with XLD with a review of the literature, to better understand this anomaly. The CT and MRI studied of 13 XLD patients were retrospectively evaluated. All structures of the otic capsule (OC) were subjectively and retrospectively assessed. The OC thickness and the size of the cochlea were measured and compared to the age-matched control group. Intracranial structures were also evaluated with specific attention to the hypothalamic region. All cases had bilateral IP-III anomaly, bulbous internal auditory canals (IACs), absent bony modiolus with preserved interscalar septa, intact cochleovestibular, and facial nerves. OC thickness was decreased in all cases compared to the control group (p<0.001). In XLD patients, the cochlea had decreased transverse dimension and increased height compared to the control group (p< 0.001). Five patients (38.4%) had bilateral cystic structures adjacent to the vestibule and/or semicircular canals (SCCs). Hypothalamus was thickened or had a lobular appearance in all cases (subtle in one). Additionally, hamartoma-like appearance of the hypothalamus was present in half. XLD is a rare inner ear anomaly that is frequently associated with hypothalamic malformations. The OC thickness of IP-III patients appears to be decreased with accompanying decreased transverse dimension of the cochlea which could have implications in electrode selection during cochlear implantation. Cystic /diverticular lesions surrounding the vestibule and semicircular canals are also frequently seen but a rarely reported finding.

Can Imaging Predict Hearing Outcomes in Children With Cochleovestibular Nerve Abnormalities?

To identify the imaging characteristics associated with better hearing outcomes found in cochleovestibular nerve (CVN) abnormalities treated with hearing aids and/or cochlear implantation (CI). Retrospective review. A retrospective review was undertaken of 69 ears with CVN abnormalities seen on magnetic resonance imaging (MRI) treated at a tertiary referral academic center analyzing the clinical features, imaging characteristics, and hearing data. We searched for associations among the hearing and imaging data, hypothesizing that the imaging data was not a good indicator of hearing function. In univariable analysis of all those who underwent aided testing (hearing aid and CI), health status (P= .016), internal auditory canal (IAC) midpoint diameter (P< .001), and number of nerves in the IAC (P< .001) were predictors of positive hearing outcome. Modiolar abnormalities, cochlear aperture diameter, cochlear malformations, vestibular malformations, and nerves in the cerebellar cistern did not predict hearing outcome (P= .79, .18, .59, .09, .17, respectively). For patients who received CI, health status (P= .018), IAC midpoint (P= .024), and number of nerves in the IAC (P= .038) were significant. When controlling for health status, IAC midpoint diameter (P< .001) and number of nerves in the IAC (P< .001) remained significant. In our cohort, one out of the eight ears (13%) with Birman class 0 or 1 exhibited responses to sound compared to nine out of 13 ears (70%) with Birman class 2-4. Current imaging modalities cannot accurately depict the status of the cochleovestibular nerve or predict a child's benefit with a CI. Cochlear implantation should be considered in children with abnormal cochleovestibular nerves. 3 Laryngoscope, 132:S1-S15, 2022.

Клінічний випадок природженої CMV-нейроінфекції в дитини з вибірковим дефіцитом IgA

Вважають, що природжена CMV-інфекція має місце щонайменше у 1 % немовлят, хоча результати останніх клінічних досліджень вказують на питому вагу зазначених уражень на рівні 8 % випадків серед усіх новонароджених. Тяжкість клінічних симптомів ураження нервової системи CMV-етіології залежить від терміну внутрішньоутробного інфікування. При ранньому інфікуванні, протягом першого триместру внутрішньоутробного періоду, коли відбувається закладка головного мозку, розвиваються тяжкі вади центральної нервової системи, включаючи аненцефалію, поренцефалію, шизенцефалію, лісенцефалію, мікрополігірію та пахігірію. При більш пізньому зараженні, протягом 2–3-го триместру вагітності, відзначають більш легкі прояви: вентрикуломегалію, порушення мієлінізації білої речовини мозку, кісти в полюсах скроневих часток, гіпогенезію мозолистого тіла, перивентрикулярні кальцифікати та ураження кохлеовестибулярних нервів. В статті наведено історію хвороби хлопчика віком 5 років з типовими клініко-інструментальними ознаками природженої CMV-інфекції. У дитини відзначались глибокий спастичний тетрапарез, тяжка затримка психомовленнєвого розвитку, рефрактерний епілептичний синдром із поліморфними нападами, порушення роботи тазових органів, унеможливлення самостійного пересування. МРТ головного мозку демонструвала типові радіологічні ознаки природженої CMV-інфекції: кортикальну атрофію, вентрикуломегалію, перивентрикулярний гліоз, поля демієлінізації в білій речовині півкуль, кісти в полюсах скроневих часток, гіпоплазію мозолистого тіла. Протягом періоду новонародженості відзначалися специфічні IgM до СMV у сироватці крові. ПЛР лейкоцитів крові виявила ДНК СMV у дитини в 5-річному віці на момент звернення в клініку за медичною допомогою. Ця інфекція призводила до генералізованої лімфаденопатії, гепатоспленомегалії, тромбоцитопенії та лімфомоноцитозу. Оцінка імунного статусу показала наявність вибіркового дефіциту IgA, з чим пов’язали розвиток цієї опортуністичної інфекції. Обговорені типові помилки при клінічному веденні дітей із природженою CMV-інфекцією та шляхи їх уникнення.

Incomplete Partition type I: Radiological Evaluation of the Temporal Bone

Objective: Incomplete partition type I is an uncommon congenital anomaly of the inner ear, characterized by typical cystic cochleovestibular appearance. Incomplete partition type I was firstly defined as cystic cochlea and vestibule without large vestibular aqueduct; however, large vestibular aqueduct and/or enlarged endolymphatic duct could rarely be seen in incomplete partition type I anomaly. Correct diagnosis of the type of cochlear malformation and differentiation of incomplete partition type I is necessary for patient management and surgical approach. Our aim was to document the temporal bone imaging findings in a series of patients with incomplete partition type I.&#x0D; Materials and Methods: CT (n=85) and/or MRI (n=80) examinations of 99 ears in 59 incomplete partition type I patients were retrospectively evaluated. All structures of the otic capsule were retrospectively assessed. The appearances of cochlea and vestibule, vestibular aqueduct/endolymphatic duct, semicircular canals were qualitatively evaluated by an experienced neuroradiologist. The vertical dimension of vestibular aqueduct and/or endolymphatic duct (from the point where the duct arises from the vestibule) was measured on CT/MRI. Anterior-posterior diameter of the internal acoustic canal and the diameter of cochlear aperture were measured on CT. The cochleovestibular nerves were evaluated on sagittal-oblique high T2-weighted imaging.&#x0D; Results: All 99 ears had defective partition with unpartitioned cochlear basal turn and absent interscalar septae, separated but cystic cochlea. The vestibule was enlarged in all ears except one. Semicircular canals were usually dysplastic (92.9%). A total of 35 incomplete partition type I ears (35.3%) had large vestibular aqueduct and/or enlarged endolymphatic duct. Internal acoustic canal was wide in 21% of ears. Cochlear aperture was wide in 5.9% of ears. Cochlear nerve was either hypoplastic or aplastic in about a quarter of incomplete partition type I ears.&#x0D; Conclusion: In up to one-third of incomplete partition type I patients, an associated large vestibular aqueduct /endolymphatic duct could be seen accompanying typical inner ear findings. Although the cochlear nerves are normal in the majority of cases, auditory brainstem implantation may be necessary in certain cases of incomplete partition type I anomaly.

CLINICAL VALUE OF VASCULAR COMPRESSION AT THE CEREBELLOPONTINE ANGLE

Purpose:To evaluate the vestibulocochlear symptoms and audiological findings in patients with radiologically detected vascular compression of the abnormally localized vertebral artery at the cerebellopontine angle. Materials and Methods: Patients with magnetic resonance imaging (MRI) with an image of vascular compression due to an angulated or lateralized vertebral artery included. The degree of vascular compression based on imaging was noted by the radiologist. The patients’ medical records were independently reviewed and the presence of tinnitus, hearing loss, vertigo and the results of mean hearing thresholds on pure tone average (PTA), speech discrimination (SD) mean scores, V latency, and I-V interpeak latency on auditory brainstem responses (ABR) were noted. Results: The close relationship was detected in 28 MR scans. The degree of compression was categorized as point compression on the nerve in 5 patients, longitudinal compression on the nerve in 17, and contact as vascular loop or indentation in the nerve in 6. There were no differences between vascular compression of vertebral artery and ipsilateral symptom patterns in all groups. In the groups, ipsilateral results of PTA, SD and ABR were found abnormal compared with contralateral results but failed to show statistical significance. Conclusion: The results suggest that there was no significant clinical value of vascular compression of the vertebral artery on the cochleovestibular nerve. In patients with unexplained hearing loss, dizziness and tinnitus complaints, deciding on the cause of compression of the vertebral artery at the cerebellopontine angle and discussing the necessity of decompression surgery will only be possible with larger patient series.

Is the cochleovestibular nerve function affected in patients with hemifacial spasm?

Hemifacial spasm (HFS) is a motor disorder caused by the vascular compression of the facial nerve in the posterior fossa. The cochleovestibular nerve is close to the facial nerve and shares the same entry to the periphery, also has disorders caused by vascular compression. We evaluated the cochleovestibular nerve function in patients with HFS based on the hypothesis that vascular compression, which causes HFS, can also affect the nearby cochleovestibular nerve function. The medical charts of 49 patients with surgically confirmed HFS were reviewed retrospectively. The results of the pure-tone threshold, auditory brainstem response (ABR), video head impulse test (vHIT), and magnetic resonance imaging were analyzed. In each patient, the HFS side and the unaffected side were compared in the paired manner. The anterior inferior cerebellar artery was the major offending vessel (69.4%). There were no significant differences in the pure-tone threshold, properties of ABR waves, and vHIT gain. There was no evidence of cochleovestibular nerve compression syndrome in all patients. The angulation of the nerve by the offending vessel was more frequently identified in the HFS side than in the unaffected side (p = 0.040). The effect of HFS on cochleovestibular nerve function is limited.

Fluorescent Detection of Vestibular Schwannoma Using Intravenous Sodium Fluorescein In Vivo.

Vestibular schwannoma (VS) are intracranial tumors caused by merlin deficiency. Sodium fluorescein (SF) is a fluorescent compound that accumulates in various intracranial tumors, causing tumors to emit green fluorescence after blue light excitation. Intravenous SF preferentially deposits in VS, helping surgeons differentiate tumor from surrounding tissue. Merlin-deficient Schwann cells were grafted onto cochleovestibular nerves of immunodeficient rats. Rats were randomized to receive SF (7.5 mg/kg; n = 5) or saline (n = 3). Tissues were harvested at 1 hour and photographed in white and blue light. Sixteen surgeons identified and marked the tumor-tissue interfaces on images. Fluorescence was measured on tissue specimens using the IVIS imaging system and on tissue cross-sections obtained with confocal microscopy. Western blot was performed to measure levels of organic anion transporting polypeptide (OATP), a drug transporter specific for SF. Under blue light, tumors from SF rats demonstrated bright green fluorescence under direct visualization, higher fluorescence measurements on tissue specimens (p < 0.001), and more SF deposition on tissue cross-sections (p < 0.001), when compared with surrounding tissues and placebo rats. Surgeons were better able to distinguish the tumor-tissue interfaces in SF rats. Furthermore, the expression level of OATP1C1 was significantly higher in tumors than in surrounding tissues (p < 0.0001). In a xenograft model of VS, intravenous SF preferentially deposits in tumors, compared with normal surrounding tissue. Under blue light, tumors emit an intense green fluorescence that can help surgeons differentiate tumor from critical structures nearby, which may improve clinical outcomes in complicated VS surgery.

A Study of Outcome of Pediatric Cochlear Implantation in Patients with Cochleovestibular Nerve Deficiency.

A cochleovestibular nerve deficiency (CVND) could compromise stimulation of nerve by electrical pulses delivered from a cochlear implant, thereby hindering activity along auditory pathway. The evaluation of children with congenital hearing loss with a high-resolution magnetic resonance imaging is presently the investigative modality of choice to diagnose CVND. The aim of this study was to determine the outcomes in pediatric cochlear implant recipients with a diagnosis of CVND. The objectives included (1) to study the prevalence of CVND among children with prelingual congenital severe to profound hearing loss; (2) to assess post cochlear implantation (CI) outcomes in children with CVND using categories of auditory performance (CAP), speech intelligibility rating (SIR), and cortical auditory evoked potentials (CAEPs); and (3) to propose a management protocol for these children. All CI procedures performed during the study period in children 5 years or younger were included in study. All patients who were older than 5 years or had syndromic associations, multiple disabilities, second side or revision CI were excluded from the study. Children with unilateral cochleovestibular nerve aplasia and all other cases of CVND (type IIa and IIb) were advised to undergo CI on side with more radiologically robust nerve and/or cochlea anatomy. Children with bilateral CVND were included in group A, and age-matched cochlear implant candidates with normal cochleovestibular nerve anatomy were included in group B for statistical comparison of outcomes. In group A, post CI CAP and SIR, CAEP amplitude and latency at 12 months showed statistically significant difference (p<0.05) compared with preoperative values. However, mean score of CAEP latency and amplitude and SIR score was worse for group A compared with group B at 12 months, which was statistically significant (p<0.05). This study supports the fact that CI is a viable option to be offered in children with CVND (type IIa and IIb) for the development of auditory perception and speech.

Genes Implicated in Rare Congenital Inner Ear and Cochleovestibular Nerve Malformations.

A small subset of children with congenital hearing loss have abnormal cochleovestibular nerves (i.e., absent, aplastic, or deficient cochlear nerves), with largely unknown etiology. Our objective was to investigate the underlying pathways and identify novel genetic variants responsible for cochleovestibular malformations and nerve abnormalities. It is our hypothesis that several cochleovestibular nerve abnormalities might share common causative pathways. We used a family-based exome sequencing approach to study 12 children with known rare inner ear and/or cochleovestibular nerve malformations. Our results highlight a diverse molecular etiology and suggest that genes important in the developing otic vesicle and cranial neural crest, e.g., MASP1, GREB1L, SIX1, TAF1, are likely to underlie inner ear and/or cochleovestibular nerve malformations. We show that several cochleovestibular nerve malformations are neurocristopathies, which is consistent with the fact that cochleovestibular nerve development is based on otic placode-derived neurons in close association with neural crest-derived glia cells. In addition, we suggest potential genetic markers for more severely affected phenotypes, which may help prognosticate individual cochlear implantation outcomes. Developing better strategies for identifying which children with abnormal nerves will benefit from a cochlear implantation is crucial, as outcomes are usually far less robust and extremely variable in this population, and current neuroimaging and electrophysiologic parameters cannot accurately predict outcomes. Identification of a suitable treatment early will reduce the use of multiple interventions during the time-sensitive period for language development.

Is Endolymphatic Sac Surgery Beneficial For Meniere's Disease?

Is Endolymphatic Sac Surgery Beneficial For Meniere's Disease?

Labyrinthine concussion: Historic otopathologic antecedents of a challenging diagnosis.

ObjectiveThe term “labyrinthine concussion” has evolved to mean audiovestibular dysfunction in the absence of a temporal bone fracture (TBF). Despite a multitude of case descriptions of labyrinthine concussion, the precise pathophysiology remains poorly understood. Herein, we explore the historical otopathologic underpinnings of the diagnosis of labyrinthine concussion with a focus on the auditory pathway during the late 19th to the mid‐20th centuries and conclude with a discussion of its contemporary relevance.Methods and Data SourcesA review of primary and secondary medical sources written in English, German, and French on otopathology labyrinthine concussion studies from the late‐19th to the mid‐20th centuries.ResultsAround the turn of the 20th century, otopathologists identified histologic changes in the temporal bones of individuals that sustained head injury without TBFs. Based on these otopathologic findings in humans, early experiments investigating the pathophysiology of labyrinthine concussion were performed in animals through either the delivery of blows to the head or direct introduction of a pressure wave into the labyrinthine fluid. Collectively, otopathologists hypothesized that predominant mechanisms for labyrinthine concussion included inner ear hemorrhage, cochleovestibular nerve traction injury, direct damage from a labyrinthine fluid pressure wave, or vasomotor dysfunction.ConclusionHistorical study shows a variety of inner ear pathologies potentially responsible for auditory dysfunction following head injury. Understanding the history and otopathology of labyrinthine concussion may help clinicians focus on new pathways toward novel research and improved patient care.

Are Cochlear Implants a Viable Option Following Temporal Bone Fracture?

Are Cochlear Implants a Viable Option Following Temporal Bone Fracture?